The Italian Gruppo di Studio delle Piastrine developed an algorithm for classifying patients with an inherited thrombocytopenia. The initial distinction is between patients with a clinical syndrome and those without. The authors are from multiple universities in Italy.
Parameters:
(1) size of the platelets
(2) amegakaryocytic thrombocytopenia
(3) gray color to the platelets
(4) Dohle-like bodies in neutrophils
(5) red blood cell abnormalities
(6) spontaneous platelet aggregation
(7) abnormal platelet aggregation with ristocetin
(8) abnormal glycoproteins GPIb/IX/V
(9) spotty distribution of non-muscle myosin heavy chain (NMMHC) IIA in white blood cells
|
Platelet Size |
Other Finding |
Diagnosis |
|
small |
|
X-linked thrombocytopenia |
|
normal |
amegakaryocytic thrombocytopenia present |
congenital amegakaryocytic thrombocytopenia |
|
normal |
amegakaryocytic thrombocytopenia absent |
autosomal dominant thrombocytopenia |
|
large |
gray platelets |
gray platelet syndrome |
|
large |
Dohle like bodies |
MYH9-related disease |
|
large |
RBC abnormalities |
X-linked thrombocytopenia |
|
large |
spontaneous platelet aggregation |
Montreal platelet syndrome |
|
large |
increased ristocetin-induced aggregation |
platelet-type (pseudo) von Willebrand's disease |
|
large |
decreased ristocetin-induced aggregation |
homozygous Bernard Soulier syndrome |
|
large |
abnormal glycoproteins |
heterozygous Bernard Soulier Syndrome |
|
large |
spotty distribution of NMMHC IIA in WBCs |
MYH9-related disease |
|
large |
none of the above |
Mediterranean macrothrombocytopenia |
|
large |
none of the above |
macrothrombocytopenia with platelet glycophorin |
Specialty: Genetics, Hematology Oncology
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